What are the other Names for this Condition? (Also known as/Synonyms)

• Epidermal Polycystic Disease
• Multiple Sebaceous Cysts
• Sebocystomatosis

What is Steatocystoma Multiplex? (Definition/Background Information)

• Steatocystoma Multiplex is an uncommon skin condition manifesting as numerous cysts at puberty. This condition is inherited in an autosomal dominant manner in many cases. It is also known as Hereditary Epidermal Polycystic Disease
• These tiny cysts may be present on the trunk and face; sometimes, they may become infected resulting in pain and discomfort. In many children, the presence of numerous lesions widespread in the body is a cause for constant cosmetic concern
• The treatment of Steatocystoma Multiplex may involve the administration of oral medications, cryotherapy, and removal of cysts via surgery. The overall prognosis is generally good, but this may vary from one individual to another

Who gets Steatocystoma Multiplex? (Age and Sex Distribution)

• Steatocystoma Multiplex is a rare congenital disorder, although the onset of the disorder occurs during puberty (between 8 and 14 years)
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Steatocystoma Multiplex? (Predisposing Factors)

• A positive family history may be an important risk factor, since Steatocystoma Multiplex can be inherited
• Currently, no other specific predisposing factors have been identified

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Steatocystoma Multiplex? (Etiology)

• In some cases, Steatocystoma Multiplex is caused by genetic abnormalities which are passed down through families in an autosomal dominant pattern. In such cases, it is caused by mutations involving the keratin 17 (KRT17) gene
• In many cases, a family history of the condition is not observed, and Steatocystoma Multiplex occurs sporadically. When Steatocystoma Multiplex occurs randomly, mutations in the KRT17 gene have not been observed

Some research experts speculate that the condition may be a mild subtype of pachyonychia congenita, a genetic disorder of the nails and skin.

What are the Signs and Symptoms of Steatocystoma Multiplex?

The signs and symptoms of Steatocystoma Multiplex may vary among the affected children in type and severity. Even though most cases are noted at puberty, the age of onset may be variable. However, a worsening of the condition is noted during puberty.

The signs and symptoms may include:

• Presence of multiple cysts (size between 1-2 mm) typically on the chest, back, limbs, and face, among other skin sites
• The cysts may be filled with an oily fluid (white-yellow in color); occasionally, a presence of hair within the cysts may be noted
• Infected cysts may be painful and cause discomfort
• If a single cyst is noted, the condition is called steatocystoma simplex
• In Steatocystoma Multiplex, between 100 to 1,000 cysts may be observed

How is Steatocystoma Multiplex Diagnosed?

Steatocystoma Multiplex is diagnosed on the basis of the following information:

• A complete evaluation of family and personal medical history along with a thorough examination of the skin lesions by a dermatologist
• Blood tests and culture studies, if necessary
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Molecular genetic testing to identify the gene involved, if available
• Skin biopsy: A skin tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Steatocystoma Multiplex?

The complications of Steatocystoma Multiplex may include:

• Emotional stress
• Secondary bacterial and fungal infections
• Cosmetic issues from scarring on healing of the lesions
• Kidney stones may be observed in a small percentage of individuals
• Adenoma sebaceum, which is a rare skin condition presenting multiple skin tumors in a background of tuberous sclerosis (a genetic disorder)

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Steatocystoma Multiplex Treated?

There is no cure for Steatocystoma Multiplex since it is a genetic condition. Also, there are no well-defined treatments available for the condition. In many, treatment is usually given to manage the signs and symptoms and any complication that develops. This may include:

• Oral isotretinoin
• Oral antibiotic therapy
• Cyst aspiration and drainage
• Liquid nitrogen cryotherapy
• Dermabrasion
• Carbon dioxide laser therapy, especially when numerous cysts are seen
• Surgical excision and cyst removal

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

How can Steatocystoma Multiplex be Prevented?

Steatocystoma Multiplex is not currently preventable since it is a genetic disorder.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

What is the Prognosis of Steatocystoma Multiplex? (Outcomes/Resolutions)

• The prognosis of Steatocystoma Multiplex is dependent upon the severity of the signs and symptoms and associated complications, if any
• In most cases, the condition is typically asymptomatic, although the presence of a large number of cysts can cause chronic cosmetic issues

Additional and Relevant Useful Information for Steatocystoma Multiplex:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/